• Vertebrate lamins consist of two types, A and B. Mutations in this gene are associated with acquired partial lipodystrophy. (nih.gov)
  • The LEM domain is also common to two other integral proteins of the INM: lamina-associated polypeptide 2 (LAP2) and emerin. (wikipedia.org)
  • The cellular phenomena associated with laminopathy invariably show disruption of nucleoskeleton of lamina due to deregulated expression, localization, function, and interaction of mutant lamin proteins. (bvsalud.org)
  • Impaired spatial and temporal tethering of lamin proteins to the lamina or nucleoplasmic aggregation of lamins are the primary molecular events that can trigger nuclear proteotoxicity by modulating differential protein-protein interactions, sequestering quality control proteins, and initiating a cascade of abnormal post-translational modifications. (bvsalud.org)
  • Here, we review nuclear proteostasis and its deregulation in the context of lamin proteins and laminopathies. (bvsalud.org)
  • The nuclear lamina is a complex network of nuclear lamins and lamin-associated nuclear membrane proteins, which scaffold the nucleus to maintain structural integrity. (bvsalud.org)
  • In Arabidopsis thaliana, nuclear matrix constituent proteins (NMCPs) are essential components of the nuclear lamina and are required to maintain the structural integrity of the nucleus and specific perinuclear chromatin anchoring. (bvsalud.org)
  • Mutations in lamina proteins associated with various laminopathies result in the loss of organization of DNA at the nuclear periphery. (bvsalud.org)
  • The nuclear lamina consists of a two-dimensional matrix of proteins located next to the inner nuclear membrane. (nih.gov)
  • The lamin family of proteins make up the matrix and are highly conserved in evolution. (nih.gov)
  • During mitosis, the lamina matrix is reversibly disassembled as the lamin proteins are phosphorylated. (nih.gov)
  • Lamin proteins are thought to be involved in nuclear stability, chromatin structure and gene expression. (nih.gov)
  • LEM domain-containing protein 3 (LEMD3), also known as MAN1, is an integral protein in the inner nuclear membrane (INM) of the nuclear envelope. (wikipedia.org)
  • Focusing on heat stress, we reveal that chromatin domains, initially tethered to the nuclear envelope, remain largely associated with CRWN1 and become scattered in the inner nuclear space. (bvsalud.org)
  • Here we report the highly flexible nature of the plant nuclear lamina, which disassembles substantially under various stress conditions. (bvsalud.org)
  • RECENT FINDINGS: Multiple studies have highlighted the role of the nuclear lamina in maintaining the integrity of the nucleus. (bvsalud.org)
  • Chromosomal aneuploidies induced upon Lamin B2 depletion are mislocalized in the interphase nucleus. (nih.gov)
  • At the nuclear periphery, suppressed chromatin overlapping with repetitive sequences and inactive protein-coding genes are enriched. (bvsalud.org)
  • On the basis of these observations in Arabidopsis, and given the role of NMCP genes (CRWN1 and CRWN4) in organizing chromatin positioning at the nuclear periphery, one can expect considerable changes in chromatin-nuclear lamina interactions when the global chromatin organization patterns are being altered in plants. (bvsalud.org)
  • This gene encodes a B type nuclear lamin. (nih.gov)
  • Clearly, laminopathic cells exhibit moderate to high nuclear proteotoxicity, raising the question of whether an imbalance in nuclear proteostasis is involved in laminopathic diseases, particularly in diseases of early aging such as HGPS and laminopathy-associated premature aging. (bvsalud.org)
  • Lamin B2 contributes to the proliferation of bladder cancer cells via activating the expression of cell division cycle‑associated protein 3. (nih.gov)
  • Lamin B2 promotes the malignant phenotype of non-small cell lung cancer cells by upregulating dimethylation of histone 3 lysine 9. (nih.gov)
  • The lamina also has a critical role in 3D genome organization. (bvsalud.org)
  • The role of lamin B2 in human diseases. (nih.gov)
  • The results highlight the importance of mid-SUNs as functional components of the ER and outer nuclear membrane. (brookes.ac.uk)
  • Many tools were designed to delimit objects in 2D and eventually in 3D, to define their shapes, their number or position in nuclear space. (brookes.ac.uk)
  • LEM domain-containing protein 3 (LEMD3), also known as MAN1, is an integral protein in the inner nuclear membrane (INM) of the nuclear envelope. (wikipedia.org)
  • We explored nine fetuses/newborns children with RD. Two were found to have an heterozygous splicing mutation in the LMNA gene, leading to the complete or partial loss of exon 11 in mRNAs encoding Lamin A and resulting in a truncated Prelamin A protein. (nih.gov)
  • and directly interacts with the nuclear lamina protein Lamin B1 and binds to lamin-associated domains (LADs) on chromatin. (acancerjourney.info)
  • demonstrated that PBC sera staining the nuclear membrane reacted with a 200 kDa nuclear protein. (medscape.com)
  • identified this protein as gp210 nuclear pore protein. (medscape.com)
  • The results highlight the importance of mid-SUNs as functional components of the ER and outer nuclear membrane. (brookes.ac.uk)
  • Keeping that in mind, throughout new throughout vitro types, lower lamin The term associated with superior cell migration along with invasiveness and, within Small molecule library vivo, with an elevated metastatic insert. (compound7ninhibitor.com)
  • Alternatively, overexpression associated with lamin The rescued LINC complicated business, hence decreasing YAP/TAZ atomic recruiting and also protecting against mobile invasiveness. (compound7ninhibitor.com)
  • Heart failure induces significant changes in nuclear pore complex of human cardiomyocytes. (nih.gov)
  • Lipidated LC3-II can Gefarnate be involved with mediating Lamin B1 discussion (Fig. prolonged and 1d Data Fig. 1e-g) as well as the LC3 G120A lipidation lacking mutant demonstrated impaired binding to Lamin B1 (Fig. 1f). (acancerjourney.info)
  • Macroautophagy (hereafter known as autophagy) is a catabolic membrane trafficking process that degrades a variety of cellular constituents and is associated with human diseases1-3. (acancerjourney.info)
  • Lamin orthologs are absent from yeast, and it is not known whether a structural or functional equivalent of the lamina exists in S. cerevisiae . (biomedcentral.com)
  • Alterations in the structure, organization, and composition of the nuclear lamina are likely to account for aberrant shapes of the nuclei of malignant cells, as well as the distinct nuclear morphology of neutrophils. (biomedcentral.com)
  • Inhibiting autophagy or the LC3-Lamin B1 interaction prevents activated Ras-induced Lamin B1 loss and attenuates oncogene-induced senescence in primary human cells. (acancerjourney.info)
  • Antibodies against nuclear lamins (reported in approximately 2% of PBC patients), and those directed against LAP (reported in approximately 16%) were also detected in patients with other autoimmune diseases. (medscape.com)